Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Clinical outcome of acute necrotizing encephalopathy in related to involving the brain stem of single institution in Korea

  • Lee, Cha Gon (Department of Pediatrics, Eulji General Hospital) ;
  • Kim, Ji Hye (Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Munhyang (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Jeehun (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 투고 : 2013.06.26
  • 심사 : 2014.02.02
  • 발행 : 2014.06.10
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초록

Purpose: Acute necrotizing encephalopathy (ANE) is a fulminant disease of the brain characterized by bilateral thalamic lesions, and is prevalent among children in East Asia. The prognosis of ANE is usually poor with a high mortality rate and neurological sequelae. This study aimed to delineate the clinical characteristics and prognostic factors of ANE. Methods: We retrospectively analyzed clinical data of 399 pediatric patients with encephalitis who were admitted to Samsung Medical Center from December 1998 to March 2011. We enrolled ten patients (11 cases) with ANE and analyzed their demographic, clinical, and neuroimaging data. The location and extent of the brain regions were checked based on fluid-attenuated inversion recovery, T1-, and T2-weighted imaging findings; the presence of contrast enhancement, restricted diffusion, and hemorrhage. Results: Ten patients were identified, including one patient with two episodes. The median age of onset was 1.5 years (0.4-8.4 years). The mortality rate was 40%, and only 30% of patients survived without neurological sequelae. The definite involvement of the brainstem on brain magnetic resonance imaging was significantly correlated with mortality (P =0.04). Conclusion: Broad and extensive brainstem involvement suggested the fulminant course of ANE. Early diagnosis of ANE before brainstem involvement, through careful identification of symptoms of brain dysfunction, may be the best way to achieve better neurological outcomes.

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참고문헌

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피인용 문헌

  1. Specific HLA genotypes confer susceptibility to acute necrotizing encephalopathy vol.17, pp.6, 2016, https://doi.org/10.1038/gene.2016.32
  2. Acute Necrotizing Encephalopathy of Childhood Secondary to Dengue Infection: A Case Report from Pakistan vol.12, pp.2, 2014, https://doi.org/10.4103/jpn.jpn_186_16
  3. Acute Necrotizing Encephalopathy Associated with Influenza A vol.60, pp.1, 2014, https://doi.org/10.1080/21646821.2020.1725864
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  6. Relevance of CSF, Serum and Neuroimaging Markers in CNS and PNS Manifestation in COVID-19: A Systematic Review of Case Report and Case Series vol.11, pp.10, 2014, https://doi.org/10.3390/brainsci11101354